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We present the clinical case of a 79 year-old patient with hypertension, treated with anticoagulants and known background of right renal angiomyolipoma that is admitted to the emergency ward due to pain and mass in right flank. The CT revealed retroperitoneal haematoma, active renal intratumoral bleeding and hemoperitoneum. A total emergency nephrectomy was carried out. The patient presented an upper intestinal fistula and died on the sixth day after surgery.

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